Splenic marginal zone lymphoma (SMZL)

What is splenic marginal zone lymphoma?

Splenic marginal zone lymphoma (SMZL) is a rare type of non-Hodgkin lymphoma (NHL).

It belongs to a group of non-Hodgkin lymphomas called marginal zone lymphomas (MZL). There are 3 types:

splenic marginal zone lymphoma (SMZL)
extranodal MZL known as mucosa-associated lymphoid tissue (MALT) lymphoma
nodal marginal zone lymphoma (NMZL).

Your doctor may talk to you about the other marginal zone lymphomas before it is clear which type of MZL you have.

SMZL develops when B-cells become abnormal (cancerous). B-cells are white blood cells that normally help fight infection. They are sometimes called B-lymphocytes.

The abnormal B-cells (lymphoma cells) usually build up in the spleen, bone marrow and blood.

Symptoms of splenic marginal zone lymphoma

Many people will have no symptoms when SMZL is first diagnosed. It is sometimes discovered during an examination of your tummy (abdomen). It can also be discovered when blood tests are done for some other reason.

The spleen may become bigger if lymphoma cells build up there. This is called splenomegaly. It can cause discomfort or pain in the upper part of the tummy.

Some people with SMZL will have a low number of blood cells in their blood. This is usually because an enlarged spleen may remove too many blood cells. It can also happen if lymphoma cells build up in the bone marrow. These cells take up space, which makes it difficult for the bone marrow to make enough normal blood cells.

If you have too few blood cells, you may:

feel tired
become breathless when moving around
bruise or bleed easily
be more at risk of infection.

Sometimes the lymphoma cells make an abnormal protein called a globulin or a paraprotein. This is found in the bloodstream. This protein can make the blood thicker and more slow-moving than normal. This can cause:

headaches
confusion
nosebleeds
blurred vision.

B symptoms

Some people also have symptoms that doctors call B symptoms. These can include:

drenching night sweats which require a change of nightwear and bed covers
high temperatures (fevers) with no obvious cause
unexplained weight loss.

Knowing if you have any B symptoms will help your doctor to stage the lymphoma and plan your treatment.

Causes and risk factors of splenic marginal zone lymphoma

The causes of splenic marginal zone lymphoma (SMZL) are mostly unknown. It is sometimes linked to long-term infection with the hepatitis C virus (HCV). But it is important to remember that most people with HCV will not develop lymphoma.

SMZL can affect people of all ages, but is most common in people over 70.

Like other cancers, SMZL is not infectious. It cannot be passed on to other people.

We have more information about causes and risk factors of lymphoma.

Diagnosis of splenic marginal zone lymphoma

SMZL can be diagnosed through a combination of:

Rarely, SMZL is diagnosed after an operation to remove the spleen.

Other tests may be done to check for signs of infection with hepatitis C virus.

You can read more about further tests you may have in our information about non-Hodgkin lymphoma.

Waiting for test results can be difficult. You may find it helpful to talk to your family, friends or specialist nurse.

The stages and grades of splenic marginal zone lymphoma

Your test results will help your doctors find out how many areas of your body are affected by lymphoma and where these areas are. This is called staging.

Lymphomas are also grouped as either low-grade or high-grade. Low-grade lymphomas are usually slow growing. High-grade lymphomas usually grow more quickly. Splenic marginal zone lymphoma (SMZL) is a low-grade lymphoma and normally develops slowly. Sometimes it can change (transform) to a high-grade lymphoma.

Knowing the stage and grade of the lymphoma helps your doctor plan the right treatment for you.

We have more information about the stages and grades of non-Hodgkin lymphoma.

Treatment for splenic marginal zone lymphoma

A team of specialists will meet to discuss the best possible treatment for you. They are called a multidisciplinary team (MDT).

Your doctor, cancer specialist or nurse will explain the different treatments and their side effects to you. They will also talk to you about things to consider when making treatment decisions.

Treatment for splenic marginal zone lymphoma (SMZL) depends on the stage of the lymphoma and whether you have symptoms that are causing problems.

SMZL usually develops slowly. Some people may not need treatment for months, or years. During this time, they will have regular check-ups to monitor the lymphoma. This is called watch and wait.

If treatment is needed, the aim is to control the lymphoma for as long as possible. This is called remission.

The most common treatments for SMZL are:

Immunotherapy and chemotherapy
SMZL is often treated with the immunotherapy drug rituximab in combination with chemotherapy. This is called chemoimmunotherapy. Sometimes rituximab is used on its own. Chemoimmunotherapy can often get the lymphoma into remission. Commonly used combinations of chemoimmunotherapy include:

R-CVP

R-CHOP

R-chlorambucil – rituximab and chlorambucil

R-bendamustine – rituximab and bendamustine.
Steroids

Steroids are drugs that are often given with chemotherapy to treat lymphomas. They help make chemotherapy more effective. effective.
Surgery to remove the spleen (splenectomy)

Surgery to remove the spleen may sometimes be useful in this type of lymphoma. If the spleen is not too large, this operation can be done using laparoscopic surgery. This is sometimes called keyhole surgery.
Radiotherapy

Radiotherapy uses high-energy rays to destroy cancer cells, while doing as little harm as possible to normal cells. You may have low doses of radiotherapy to the spleen. This treatment may be used if the enlarged spleen is causing symptoms, but you are not well enough to have surgery or chemotherapy.

You may have some treatments as part of a clinical trial.

If SMZL comes back after treatment it is called a recurrence or relapse. When this happens, you can usually have more treatment to help control it again. We have more information about treating lymphoma that has come back.

After splenic marginal zone lymphoma treatment

You will have regular follow-up appointments after your treatment. You can talk to your doctor or nurse about any concerns you may have at these appointments. Your doctor will want to know how you are feeling, and to check you are recovering from any side effects of treatment.

Late effects

Sometimes side effects may continue or develop months or years after treatment. These are called late effects. We have more information about long-term and late effects of treatment for lymphoma.

Sex life and fertility

Cancer and its treatment can sometimes affect your sex life. There ways to improve your sexual well-being and to manage any problems.

Treatment for lymphoma may affect your fertility. If you are worried about your fertility it is important to talk with your doctor before you start treatment. We have more information about:

Well-being and recovery

Even if you already have a healthy lifestyle, you may choose to make some positive lifestyle changes after treatment.

Making small changes such as eating well and keeping active can improve your health and wellbeing and help your body recover.

Getting support

Everyone has their own way of dealing with illness and the different emotions they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse.

Macmillan is also here to support you. If you would like to talk, you can:

Call the Macmillan Support Line on 0808 808 00 00.
Chat to our specialists online.
Visit our non-Hodgkin lymphoma forum to talk with people who have been affected by non-Hodgkin lymphoma (NHL), share your experience, and ask an expert your questions.

The organisations below also offer information and support:

Blood Cancer UK

Blood Cancer UK offers support and information to people affected by blood cancers, including lymphoma.
Lymphoma Action

Lymphoma Action gives emotional support, advice and information for people with Hodgkin lymphoma or non-Hodgkin lymphoma and those close to them. It has a national network of people with lymphoma, as well as local groups. Their website has a section called trialslink where you can see information about lymphoma clinical trials.

About our information

References

Below is a sample of the sources used in our splenic marginal zone lymphoma (SMZL) information below. If you would like more information about the sources we use, please contact us at cancerinformationteam@macmillan.org.uk

National Institute of Health and Care Excellence (NICE). Blood and bone marrow cancers. NICE Pathways. Last accessed 3 December 2020.

Zucca. E, Arcani. L, Buske. C on behalf of the ESMO Guidelines Working Group. Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Published 6th January 2020. Available at: Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up - Annals of Oncology

Reviewers

This information has been written, revised and edited by Macmillan Cancer Support’s Cancer Information Development team. It has been reviewed by expert medical and health professionals and people living with cancer. It has been approved by Senior Medical Editor, Dr Anne Parker, Consultant Haematologist.

Our cancer information has been awarded the PIF TICK. Created by the Patient Information Forum, this quality mark shows we meet PIF’s 10 criteria for trustworthy health information.